A 72-year-old male referred for surgical treatment underwent chest computed tomography to further investigate a well defined mass with a maximal diameter of 3.2 cm at the left S10. There was no pathological diagnosis established by either bronchoscopic biopsy specimens or computed tomography-guided percutaneous needle biopsy. The lower lobe and mediastinal lymph nodes were excised (complete resection). The 6.5-cm encapsulated grayish mass showed extensive areas of hemorrhage and necrosis on cut surface. Results from histological and immunohistochemical study yielded a definitive diagnosis of malignant schwannoma. Primary malignant schwannomas of the lung are extremely rare and the prognosis is poor in most cases. Our patient is currently well without recurrence more than 2 years after complete resection. Complete surgical resection is the best therapeutic option for primary malignant schwannoma of the lung.